Correspondence: Anne Rod, Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

Abstract

Context : Ectopic ACTH syndrome (EAS) is principally associated with aggressive malignant tumors but also with neuroendocrine tumors of good prognosis. Recently, rare nonhepatocytic nested stromal and epithelial tumors (NSET) were characterized by their possible association with Cushing’s syndrome, which biochemical and physiopathological features were still incompletely studied.

Objective : To describe the clinical and hormonal characteristics of an EAS originating from a liver NSET, and further understand the mechanism of cortisol overproduction.

Design and setting : This is a clinical case report from the Endocrinology department of Caen University Hospital, France.

Patient and Intervention : A 17-yr old female patient was found to have a large liver NSET with mild Cushingoid clinical features, intense biological hypercortisolism but moderate ACTH secretion. Resection of the tumor was curative with a 30-months follow-up.